5-(3,3-dimethylguanidino)-2-oxopentanoate

Three steps

• pristanoyl-CoAReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + oxaloacetate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-aspartate
  2. acetyl-CoA:oxaloacetate C-acetyltransferase (thioester-hydrolysing)
  3. fatty-acid-CoA ligase

  Catalysed by AGXT2, CS, SLC27A2mitochondrial matrix, cytosolOne of 2 routes

• undecanoyl-CoAReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + oxaloacetate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-aspartate
  2. acetyl-CoA:oxaloacetate C-acetyltransferase (thioester-hydrolysing)
  3. medium-chain acyl-CoA ligase (undecylic acid)

  Catalysed by AGXT2, CS, ACSM4, ACSM1 and 4 moremitochondrial matrix, cytosolOne of 3 routes

• 3-phenyllactateReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + glyoxylate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + glycine
  2. glyoxylate + L-phenylalanine = 3-phenylpyruvate + glycine
  3. Formation of Phenyl-Lactate

  Catalysed by AGXT2, AADATcytosol

• glycodeoxycholateReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + pyruvate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-alanine
  2. N(omega),N(omega)-dimethyl-L-arginine + glyoxylate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + glycine
  3. Glycodeoxycholate amidohydrolase

  Catalysed by AGXT2cytosolOne of 2 routes

• (5Z)-tetradecenoyl-CoAReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + oxaloacetate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-aspartate
  2. acetyl-CoA:oxaloacetate C-acetyltransferase (thioester-hydrolysing)
  3. long-chain-fatty-acid-CoA ligase (physeteric acid)

  Catalysed by AGXT2, CS, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 8 routes

• (3E)-decenoyl-CoAReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + oxaloacetate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-aspartate
  2. acetyl-CoA:oxaloacetate C-acetyltransferase (thioester-hydrolysing)
  3. Fatty Acid Beta Oxidation (C12:1->C10:1), Mitochondrial

  Catalysed by AGXT2, CS, ACADM, ECHS1 and 2 moremitochondrial matrix

• N-tetradecanoylglycineReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + pyruvate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-alanine
  2. N(omega),N(omega)-dimethyl-L-arginine + glyoxylate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + glycine
  3. tetradecanoyl-CoA + glycine = N-tetradecanoylglycine + CoA + H(+)

  Catalysed by AGXT2, GLYATL2One of 2 routes

• (2R,6)-dimethylheptanoyl-CoAReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + oxaloacetate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-aspartate
  2. acetyl-CoA:oxaloacetate C-acetyltransferase (thioester-hydrolysing)
  3. acetyl-CoA C-acyltransferase (3-oxo-4(R),8-dimethyl-nonanoyl-CoA)

  Catalysed by AGXT2, CS, HADHA, ACAA2 and 1 moremitochondrial matrix

• N-eicosanoylglycinateReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + pyruvate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-alanine
  2. N(omega),N(omega)-dimethyl-L-arginine + glyoxylate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + glycine
  3. eicosanoyl-CoA + glycine = N-eicosanoylglycinate + CoA + H(+)

  Catalysed by AGXT2, BAAT

• N-docosanoylglycineReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + pyruvate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-alanine
  2. N(omega),N(omega)-dimethyl-L-arginine + glyoxylate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + glycine
  3. docosanoyl-CoA + glycine = N-docosanoylglycine + CoA + H(+)

  Catalysed by AGXT2, BAAT

• N-hexacosanoylglycineReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + pyruvate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-alanine
  2. N(omega),N(omega)-dimethyl-L-arginine + glyoxylate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + glycine
  3. hexacosanoyl-CoA + glycine = N-hexacosanoylglycine + CoA + H(+)

  Catalysed by AGXT2, BAAT

• (7Z)-hexadecenoyl-CoAReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + oxaloacetate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-aspartate
  2. acetyl-CoA:oxaloacetate C-acetyltransferase (thioester-hydrolysing)
  3. long-chain-fatty-acid-CoA ligase (7-palmitoleic acid)

  Catalysed by AGXT2, CS, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 8 routes

• tricosanoyl-CoAReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + oxaloacetate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-aspartate
  2. acetyl-CoA:oxaloacetate C-acetyltransferase (thioester-hydrolysing)
  3. long-chain-fatty-acid-CoA ligase (tricosanoic acid)

  Catalysed by AGXT2, CS, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 4 routes

• Se-methylselenocysteineReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + 2-oxobutanoate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + (2S)-2-aminobutanoate
  2. Selenocystathionine Lysteine-lyase (deaminating)
  3. L-Serine hydro-lyase (adding homocysteine)

  Catalysed by AGXT2, CTH, CBScytosol

• thromboxane B2Reach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + pyruvate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-alanine
  2. (S)-lactate + NAD(+) = pyruvate + NADH + H(+)
  3. thromboxane B2(out) + 2 (S)-lactate(in) = thromboxane B2(in) + 2 (S)-lactate(out)

  Catalysed by AGXT2, LDHA, LDHB, LDHC and 3 moreinner side of the membrane, outer side of the membraneOne of 2 routes

• 3-deoxy-D-glycero-beta-D-galacto-non-2-ulopyranosonate 9-phosphateReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + oxaloacetate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-aspartate
  2. oxaloacetate + GTP = phosphoenolpyruvate + GDP + CO2
  3. aldehydo-D-mannose 6-phosphate + phosphoenolpyruvate + H2O = 3-deoxy-D-glycero-beta-D-galacto-non-2-ulopyranosonate 9-phosphate + phosphate

  Catalysed by AGXT2, PCK1, PCK2, NANS

• thiamine(1+) diphosphateReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + pyruvate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-alanine
  2. pyruvate:thiamin diphosphate acetaldehydetransferase (decarboxylating)
  3. 2-(alpha-hydroxyethyl)thiamine-diphosphate[m] + lipoamide[m] = S-acetyldihydrolipoamide[m] + thiamin-PP[m]

  Catalysed by AGXT2, PDHA1, PDHB, PDHA2 and 1 moremitochondrial matrixOne of 2 routes